ABSTRACT
Objective: To present paediatric cases of unusual neuroinflammatory conditions encountered during the COVID-19 pandemic in Trinidad & Tobago. Methods: Retrospective study design. Inpatient paediatric patients (aged 0-16 years) hospitalized for neurological complaints from June 2020 - August 2021 at EWMSC. Outcome measures were age at presentation, sex, ethnicity, diagnosis, radiological findings, blood/CSF findings, COVID-19 PCR and antibodies testing, treatment, outcomes and other systems involved. Results: Twenty (20) patients (aged 4-months-old to 15-years-old) had documented neurological involvement. 50% had a diagnosis of ADEM/ADS/AHNE;45% had a diagnosis of either CNS vasculitis (n=3), autoimmune encephalitis (n=3) or GBS (n=3);5% had a diagnosis of acute COVID-19 encephalitis. 70% were of African descent. The youngest age group (0-4 years) (n=11) constituted more males (82%) whereas the eldest age group (10-15 years) (n=3) were all females. Neuroimaging findings were corpus callosal lesions;deep white matter T2 hyperintensities;cerebellar involvement;area postrema and brainstem/C-spine involvement;microhaemorrhages and necrotizing/haemorrhagic lesions (peripheral/central). 70% of patients were either SARS-CoV-2 PCR or COVID-19 antibodies positive. Other systems were involved in 40% to 62.5% (n=5) had cardiac involvement (myocarditis, coronary arteries dilatation, valve regurgitation) and 37.5% (n=3) had pancreatic involvement (autoimmune pancreatitis, type 1 diabetes mellitus). Treatment modalities for CNS manifestations (n=17) were clinically based - 24% (n=4) 3rd line treatment, 29% (n=5) 2nd line treatment, 41% (n=7) 1st line treatment and 6% (n=1) requiring no treatment. All 3 patients with a diagnosis of GBS responded appropriately to IVIG. Developmental outcomes were worst in patients with a diagnosis of autoimmune encephalitis. Conclusion: We have had an explosion of neuro-inflammatory cases since the COVID-19 pandemic began. The range of neuroradiological diagnoses and other systemic involvement (including criteria for PIMS) are interesting, alluding to a neuroinflammatory mechanism. Effects on long-term sequelae and developmental outcomes are concerning in some cases, however, still unknown at this stage.
ABSTRACT
Background Sneddon syndrome is a rare, progressive small and medium-vessel vasculopathy characterized by the clinical occurrence of livedo racemosa and ischaemic cerebrovascular events. Objectives We present the clinical course and management of this rare condition. Methods An 18-year-old female of East Indian descent presented with the following: CNS involvement: Recurrent Cerebral Venous Sinus Thromboses + Cognitive impairment - Skin: Livedo reticularis rash - Eyes: Bilateral optic nerves atrophy - CVS: Mild to moderate LAD dilation (16/12/2020) - MS: Polyarticular arthritis She first presented at 8-years-old with new-onset squint, ataxia and fever and was treated as culture-negative meningitis (CSF white cells 1238 neutrophils, CSF protein 110mg/ dL). At 16-years-old, she presented with expressive aphasia, headache, fever, left earache and was treated as acute mastoiditis. Regarding the recurrent presentations of CVST's there was involvement of left transverse sinus (untreated 2 years ago), then over a 1-month period despite anticoagulation, right transverse sinus with extension into the right sigmoid sinus and left straight and posterior superior sagittal sinus. She presented with headache, vomiting and new-onset seizures (GTCS) prior to these presentations. An extension of this clot after being non-compliant with low-molecular weight heparin for 1 week resulted in a venous infarct with surrounding oedema. She presented with progressive right-sided weakness and expressive aphasia and within 24 hours, encephalopathic with decerebrate posturing in subclinical status epilepticus. She was managed in PICU for refractory status epilepticus and raised intracranial pressure. There is also a family history of early stroke (maternal cousin with CVST at 8yo on long-term anticoagulation). Results Her inflammatory markers continued to increase despite antibiotic coverage at meningitic doses and she was treated for a CNS vasculitis/Catastrophic Antiphospholipid syndrome with IV anticoagulation (UFH), high-dose steroids then IVIG. Her neurological state gradually improved (coma -> vegetative state -> minimally conscious -> conscious). This was followed by Rituximab therapy 375 mg/m2 weekly x4. Her neurological function gradually improved as she was able to verbally communicate and developed anti-gravity movement of the right side. Hypertension and fever also settled, and inflammatory markers steadily decreased post treatment. Investigations: ANA, dsDNA, ENA negative pANCA borderline positive but MPO, PR3 Antibodies negative Anticardiolipin antibodies negative (on warfarin) Infectious screen (HIV, Hepatitis, COVID-19 serology, Mantoux test, CSF Acid-fast bacilli) - negative CSF cell count - 33 white cells (neutrophils), protein 183mg/dL Skin biopsy report (26/11/2020) - Neutrophils, lymphocytes of leucocytoclasis seen in vessels of dermis. Thrombosis of fibrinoid necrosis of vessel walls and extravasated red blood cells also seen - Obstructive vasculopathy for clinical correlation;Possible Sneddon syndrome/Antiphospholipid syndrome Conclusions There are few case reports describing the clinical course and treatment of this rare syndrome. In our case, treatment for Catastrophic Antiphospholipid syndrome (steroids, IVIG, rituximab and anticoagulation) was beneficial in improving the clinical outcome.